Henein Michael Y, Pilebro Björn, Lindqvist Per
Department of Public Health and Clinical Medicine, Umeå University, Umeå, Sweden.
Departments of Diagnostics and Intervention, Clinical Physiology, Umeå University, 907 37 Umeå, Sweden.
Eur Heart J Imaging Methods Pract. 2024 Oct 8;2(3):qyae105. doi: 10.1093/ehjimp/qyae105. eCollection 2024 Jul.
Echocardiography plays an important role in suspecting the presence of transthyretin cardiomyopathy (ATTR-CM) in patients with heart failure, based on parameters proposed as 'red flags' for the diagnosis of ATTR-CM. We aimed to validate those measurements in a group of patients with ATTR-CM including ATTRv and ATTRwt.
We tested a number of echocardiographic red flags in 118 patients with confirmed diagnosis of ATTR-CM. These variables were validated against healthy controls and patients with heart failure with left ventricular hypertrophy (LVH) but not ATTR-CM. The red flag measures outside the proposed cut-off values were also revalidated. In ATTR-CM, all conventional echocardiographic parameters were significantly abnormal compared with controls. Comparing ATTR-CM and LVH, LV wall thickness, LV diameter, E velocity, and relative apical sparing (RELAPS) were all different. Eighty-three per cent of ATTR-CM patients had RELAPS > 1.0, 73% had relative wall thickness (RWT) > 0.6, 72% had LVEF > 50%, 24% had global longitudinal strain (GLS) > -13%, 33% had LVEF/GLS > 4, and 54% had increased left atrial volume index (>34 mL/m). Forty per cent of ATTR-CM patients had stroke volume index < 30 mL/m and 52% had cardiac index < 2.5 L/min/m. RELAPS, LVEF, and RWT, in order of accuracy, were the three best measures for the presence ATTR-CM in the patient cohort, who all had thick myocardium. The concomitant presence of the three disturbances was found in only 50% but the combination of RELAPS > 1.0 and RWT > 0.6 was found in 72% of the patient cohort.
Increased relative apical sparing proved the most accurate independent marker of the presence of ATTR-CM followed by normal LV ejection fraction and then increased relative wall thickness. The other proposed red flags for diagnosing ATTR-CM did not feature as reliable disease predictors.
基于被提议作为诊断转甲状腺素蛋白心肌病（ATTR-CM）“红旗”指标的参数，超声心动图在怀疑心力衰竭患者存在ATTR-CM时发挥着重要作用。我们旨在对一组包括ATTRv和ATTRwt的ATTR-CM患者的这些测量指标进行验证。
我们在118例确诊为ATTR-CM的患者中测试了多项超声心动图红旗指标。这些变量与健康对照以及患有左心室肥厚（LVH）但无ATTR-CM的心力衰竭患者进行了验证。超出提议临界值的红旗指标也进行了重新验证。在ATTR-CM中，与对照组相比，所有传统超声心动图参数均显著异常。比较ATTR-CM和LVH，左心室壁厚度、左心室直径、E速度和相对心尖保留（RELAPS）均有所不同。83%的ATTR-CM患者RELAPS>1.0，73%的患者相对壁厚度（RWT）>0.6，72%的患者左心室射血分数（LVEF）>50%，24%的患者整体纵向应变（GLS）>-13%，33%的患者LVEF/GLS>4，54%的患者左心房容积指数增加（>34 mL/m）。40%的ATTR-CM患者每搏输出量指数<30 mL/m，52%的患者心脏指数<2.5 L/min/m。按准确性排序，RELAPS、LVEF和RWT是该心肌增厚患者队列中存在ATTR-CM的三项最佳测量指标。仅50%的患者同时存在这三种异常，但72%的患者队列中发现了RELAPS>1.0和RWT>0.6的组合。
相对心尖保留增加被证明是ATTR-CM存在的最准确独立标志物，其次是正常左心室射血分数，然后是相对壁厚度增加。其他提议的用于诊断ATTR-CM的红旗指标并非可靠的疾病预测指标。
